IV Trigeminal autonomic cephalalgias CQ IV-1 How are trigeminal autonomic cephalalgias classified and typed? Recommendation The International Classification of Headache Disorders 3rd Edition (beta version; ICHD-3 beta) classifies cluster headache together with related diseases under “Trigeminal autonomic cephalalgias”. Furthermore, “Trigeminal autonomic cephalalgias” is further divided into five subtypes: cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks, hemicrania continua and probable trigeminal autonomic cephalalgia. Grade A Background and Objective The objective of this section is to classify Trigeminal“ autonomic cephalalgias” according to the International Classification of Headache Disorders 3rd Edition (beta version; ICHD-3 beta).1)2) Comments and Evidence Cluster headache and related diseases are characterized by short-lasting, unilateral headache attacks accompanied by cranial parasympathetic autonomic symptoms including conjunctival injection, lacrimation, and rhinorrhea. These syndromes support the involvement of trigeminal-parasympathetic reflex activation, and ICHD-3 beta introduces the concept of trigeminal autonomic cephalalgias (TACs) (Table 1). TACs comprise the following subtypes: 3.1 cluster headache, 3.2 paroxysmal hemicrania, 3.3 short-lasting unilateral neuralgiform headache attacks, 3.4 hemicrania continua, and 3.5 probable trigeminal-autonomic cephalalgia. Table 1. Classification of “3.Trigeminal autonomic cephalalgias” 3.1 Cluster headache 3.1.1 Episodic cluster headache 3.1.2 Chronic cluster headache 3.2 Paroxysmal hemicrania 3.2.1 Episodic paroxysmal hemicrania 3.2.2 Chronic paroxysmal hemicrania (CPH) 3.3 Short-lasting unilateral neuralgiform headache attacks 3.3.1 Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) 3.3.1.1 Episodic SUNCT 3.3.1.2 Chronic SUNCT 3.3.2 Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) 3.3.2.1 Episodic SUNA 3.3.2.2 Chronic SUNA 3.4 Hemicrania continua 3.4.1 Hemicrania continua, remitting subtype 3.4.2 Hemicrania continua, unremitting subtype 3.5 Probable trigeminal autonomic cephalalgia 3.5.1 Probable cluster headache • References 1) Headache Classification Committee of the International Headache Society. The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia. 2013; 33: 629-808. 2) International Headache Classification Promotion Committee of Japanese Headache Society (translator): International Classification of Headache Disorders 3rd Edition (beta version). Igakushoin, 2014. (In Japanese) • Search terms and secondary sources • Search database: Ichushi Web for articles published in Japan (2011/12/21) Cluster headache 585 Cluster headache and classification 98 • Search database: PubMed (2011/12/21) Cluster headache 2865 Cluster headache and classification 307 172 Clinical Practice Guideline for Chronic Headache 2013 CQ IV-2 How are trigeminal autonomic cephalalgias diagnosed? Recommendation Trigeminal autonomic cephalalgias are diagnosed according to the International Classification of Headache Disorders 3rd Edition (beta version; ICHD-3 beta). Grade A Background and Objective This section describes the diagnostic criteria of the various diseases included in trigeminal“ autonomic cephalalgias” as provided by the International Classification of Headache Disorders 3rd edition (beta version; ICHD-3 beta). Comments and Evidence The International Classification of Headache Disorders 3rd edition (beta version; ICHD-3 beta)1)2) provides the diagnostic criteria for the headache types included in “3. Trigeminal autonomic cephalalgias” as follows: 3.1 Cluster headache • Diagnostic criteria A. At least five attacks fulfilling criteria B-D B. Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15-180 min (when untreated)1) C. Either or both of the following: 1. at least one of the following symptoms or signs, ipsilateral to the headache: a) conjunctival injection and/or lacrimation b) nasal congestion and/or rhinorrhoea c) eyelid oedema d) forehead and facial sweating e) forehead and facial flushing f) sensation of fullness in the ear g) miosis and/or ptosis 2. a sense of restlessness or agitation D. Attacks have a frequency between one every other day and 8 per day for more than half of the time when the disorder is active E. Not better accounted for by another ICHD-3 diagnosis. Note: 1. During part (but less than half) of the time-course of 3.1 Cluster headache, attacks may be less severe and/or of shorter or longer duration. 3.1.1 Episodic cluster headache • Diagnostic criteria A. Attacks fulfilling criteria for 3.1 Cluster headache and occurring in bouts (cluster periods) B. At least two cluster periods lasting from 7 days to 1 year (when untreated) and separated by pain-free remission periods of ≥1 month. 3.1.2 Chronic cluster headache • Diagnostic criteria A. Attacks fulfilling criteria for 3.1 Cluster headache, and criterion B below B. Occurring without a remission period or with remissions lasting <1 month, for at least 1 year. Chapter IV 173 3.2 Paroxysmal hemicrania • Diagnostic criteria A. At least 20 attacks fulfilling criteria B-E B. Severe unilateral orbital, supraorbital and/or temporal pain lasting 2-30 min C. At least one of the following symptoms or signs, ipsilateral to the pain: 1. conjunctival injection and/or lacrimation 2. nasal congestion and/or rhinorrhea 3. eyelid edema 4. forehead and facial sweating 5. forehead and facial flushing 6. sensation of fullness in the ear 7. miosis and/or ptosis D. Attacks have a frequency above five per day for more than half of the time E. Attacks are prevented absolutely by therapeutic doses of indomethacin1) F. Not better accounted for by another ICHD-3 diagnosis. • Note: 1. In an adult, oral indomethacin should be used initially in a dose of at least 150 mg daily and increased if necessary up to 225 mg daily. The dose by injection is 100-200 mg. Smaller maintenance doses are often employed. [In Japan, oral indomethacin is used up to a dose of 75 mg and the rectal formulation (suppository) up to 100 mg. Therefore, for differentiating indomethacin-responsive headache, if no response is observed when the oral formulation is used up to the highest dose of 75 mg and the rectal formulation (suppository) up to the highest dose of 100 mg, then the case can be evaluated as nonresponsive.] 3.3 Short-lasting unilateral neuralgiform headache attacks • Diagnostic criteria A. At least 20 attacks fulfilling criteria B-D B. Moderate or severe unilateral head pain, with orbital, supraorbital, temporal and/or other trigeminal distribution, lasting for 1-600 seconds and occurring as single stabs, series of stabs or in a saw-tooth pattern C. At least one of the following cranial autonomic symptoms or signs, ipsilateral to the pain: 1. conjunctival injection and/or lacrimation 2. nasal congestion and/or rhinorrhoea 3. eyelid edema 4. forehead and facial sweating 5. forehead and facial flushing 6. sensation of fullness in the ear 7. miosis and/or ptosis D. Attacks have a frequency of at least one a day for more than half of the time when the disorder is active E. Not better accounted for by another ICHD-3 diagnosis. 3.3.1 Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) • Diagnostic criteria A. Attacks fulfilling criteria for 3.3 Short-lasting unilateral neuralgiform headache attacks B. Both of conjunctival injection and lacrimation (tearing). 3.3.2 Short lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) • Diagnostic criteria A. Attacks fulfilling criteria for 3.3 Short-lasting unilateral neuralgiform headache attacks, and criterion B below B. Only one or neither of conjunctival injection and lacrimation (tearing). 3.4 Hemicrania continua • Diagnostic criteria: A. Unilateral headache fulfilling criteria B-D B. Present for >3months, with exacerbations of moderate or greater intensity 174 Clinical Practice Guideline for Chronic Headache 2013 C. Either or both of the following: 1. at least one of the following symptoms or signs, ipsilateral to the headache: a) conjunctival injection and/or lacrimation b) nasal congestion and/or rhinorrhoea c) eyelid edema d) forehead and facial sweating e) forehead and facial flushing f) sensation of fullness in the ear g) miosis and/or ptosis 2. a sense of restlessness or agitation, or aggravation of the pain by movement D. Responds absolutely to therapeutic doses of indomethacin1) E. Not better accounted for by another ICHD-3 diagnosis. Note: 1. In an adult, oral indomethacin should be used initially in a dose of at least 150 mg daily and increased if necessary up to 225 mg daily. The dose by injection is 100-200 mg. Smaller maintenance doses are often employed. [In Japan, oral indomethacin is used up to a dose of 75 mg and the rectal formulation (suppository) up to 100 mg. Therefore, for differentiating indomethacin-responsive headache, if no response is observed when the oral formulation is used up to the highest dose of 75 mg and the rectal formulation (suppository) up to the highest dose of 100 mg, then the case can be evaluated as nonresponsive.] 3.5 Probable trigeminal autonomic cephalalgia • Diagnostic criteria: A. Headache attacks fulfilling all but one of criteria A-D for 3.1Cluster headache, criteria A-E for 3.2 Paroxysmal hemicrania, criteria A-D for 3.3 Short-lasting unilateral neuralgiform